Skip to main content

Advertisement

SpringerLink
Log in

The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR)

  • Original article
  • Published:
Clinical and Experimental Nephrology Aims and scope Submit manuscript

Abstract

Background

In connective tissue diseases, a wide variety of glomerular, tubulointerstitial, and vascular lesions of the kidney are observed. Nonetheless, recent information is limited regarding renal lesions in connective tissue diseases, except in systemic lupus erythematosus (SLE).

Methods

In this study, we used a nationwide database of biopsy-confirmed renal diseases in Japan (J-RBR) (UMIN000000618). In total, 20,523 registered patients underwent biopsy between 2007 and 2013; from 110 patients with connective tissue diseases except SLE, we extracted data regarding the clinico-pathological characteristics of the renal biopsy.

Results

Our analysis included patients with rheumatoid arthritis (RA) (n = 52), Sjögren’s syndrome (SjS) (n = 35), scleroderma (n = 10), mixed connective tissue disease (MCTD; n = 5), anti-phospholipid syndrome (APS; n = 3), polymyositis/dermatomyositis (PM/DM; n = 1), Behçet’s disease (n = 1) and others (n = 3). The clinico-pathological features differed greatly depending on the underlying disease. The major clinical diagnosis was nephrotic syndrome in RA; chronic nephritic syndrome with mild proteinuria and reduced renal function in SjS; rapidly progressive nephritic syndrome in scleroderma. The major pathological diagnosis was membranous nephropathy (MN) and amyloidosis in RA; tubulointerstitial nephritis in SjS; proliferative obliterative vasculopathy in scleroderma; MN in MCTD. In RA, most patients with nephrosis were treated using bucillamine, and showed membranous nephropathy.

Conclusions

Using the J-RBR database, our study revealed that biopsy-confirmed cases of connective tissue diseases such as RA, SjS, scleroderma, and MCTD show various clinical and pathological characteristics, depending on the underlying diseases and the medication used.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Kronbichler A, Mayer G. Renal involvement in autoimmune connective tissue diseases. BMC Med. 2013;11:95.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Horak P, Smrzova A, Krejci K, Tichy T, Zadrazil J, Skacelova M. Renal manifestations of rheumatic diseases. A review. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2013;157:98–104.

    PubMed  Google Scholar 

  3. Kidney Disease Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl. 2012; 2:139–274.

    Article  Google Scholar 

  4. Nakano M, Ueno M, Nishi S, Shimada H, Hasegawa H, Watanabe T, et al. Analysis of renal pathology and drug history in 158 Japanese patients with rheumatoid arthritis. Clin Nephrol. 1998;50:154–60.

    CAS  PubMed  Google Scholar 

  5. Helin HJ, Korpela MM, Mustonen JT, Pasternack AI. Renal biopsy findings and clinicopathologic correlations in rheumatoid arthritis. Arthritis Rheum. 1995;38:242–7.

    Article  CAS  PubMed  Google Scholar 

  6. Maripuri S, Grande JP, Osborn TG, Fervenza FC, Matteson EL, Donadio JV, et al. Renal involvement in primary Sjögren’s syndrome: a clinicopathologic study. Clin J Am Soc Nephrol. 2009;4:1423–31.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Ren H, Wang WM, Chen XN, Zhang W, Pan XX, Wang XL, et al. Renal involvement and followup of 130 patients with primary Sjögren’s syndrome. J Rheumatol. 2008;35:278–84.

    CAS  PubMed  Google Scholar 

  8. Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, Cavazzana I, et al. Clinical and morphological features of kidney involvement in primary Sjögren’s syndrome. Nephrol Dial Transpl. 2001;16:2328–36.

    Article  CAS  Google Scholar 

  9. Shanmugam VK, Steen VD. Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol. 2012;24:669–76.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Sugiyama H, Yokoyama H, Sato H, Saito T, Kohda Y, Nishi S, et al. Japan Renal Biopsy Registry: the first nationwide, web-based, and prospective registry system of renal biopsies in Japan. Clin Exp Nephrol. 2011;15:493–503.

    Article  PubMed  Google Scholar 

  11. Sugiyama H, Yokoyama H, Sato H, Saito T, Kohda Y, Nishi S, et al. Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010. Clin Exp Nephrol. 2013;17:155–73.

    Article  PubMed  Google Scholar 

  12. Yokoyama H, Taguchi T, Sugiyama H, Sato H; Committee for the Standardization of Renal Pathological Diagnosis and for Renal Biopsy and Disease Registry in the Japanese Society of Nephrology. Membranous nephropathy in Japan: analysis of the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2012;16:557–63.

    Article  PubMed  Google Scholar 

  13. Yokoyama H, Sugiyama H, Sato H, Taguchi T, Nagata M, Matsuo S, et al. Renal disease in the elderly and the very elderly Japanese: analysis of the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2012;16:903–20.

    Article  PubMed  Google Scholar 

  14. Komatsu H, Fujimoto S, Yoshikawa N, Kitamura H, Sugiyama H, Yokoyama H. Clinical manifestations of Henoch-Schönlein purpura nephritis and IgA nephropathy: comparative analysis of data from the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol. 2016;20:552–60.

    Article  PubMed  Google Scholar 

  15. Yokoyama H, Narita I, Sugiyama H, Nagata M, Sato H, Ueda Y, et al. Drug-induced kidney disease: a study of the Japan Renal Biopsy Registry from 2007 to 2015. Clin Exp Nephrol. 2016;20:726–30.

    Article  Google Scholar 

  16. Matsuo S, Imai E, Horio M, Yasuda Y, Tomita K, Nitta K, et al. Revised equations for estimated GFR from serum creatinine in Japan. Am J Kidney Dis. 2009;53:982–92.

    Article  CAS  PubMed  Google Scholar 

  17. Kidney Disease. Improving Global Outcomes (KDIGO) CKD Work Group. KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney Int Suppl. 2013;3:1–150.

    Article  Google Scholar 

  18. Takasaki Y. Epidemiology of collagen diseases. Nihon Rinsho. 2009;67:458–62 (Japanese).

    PubMed  Google Scholar 

  19. Makino H, Yoshinaga Y, Yamasaki Y, Morita Y, Hashimoto H, Yamamura M. Renal involvement in rheumatoid arthritis: analysis of renal biopsy specimens from 100 patients. Mod Rheumatol. 2002;12:148–54.

    Article  CAS  PubMed  Google Scholar 

  20. Kitridou RC, Akmal M, Turkel SB, Ehresmann GR, Quismorio FP Jr, Massry SG. Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study. Semin Arthritis Rheum. 1986;16:135–45.

    Article  CAS  PubMed  Google Scholar 

  21. Yoshida A, Morozumi K, Takeda A, Koyama K. Nephropathy in patients with mixed connective tissue disease. Ryumachi. 1994;34:976–80. (Japanese).

    CAS  PubMed  Google Scholar 

  22. Ungprasert P, Crowson CS, Chowdhary VR, Ernste FC, Moder KG, Matteson EL. Epidemiology of mixed connective tissue disease, 1985–2014: a population-based study. Arthritis Care Res. 2016;68:1843–8.

    Article  Google Scholar 

  23. Burdt MA, Hoffman RW, Deutscher SL, Wang GS, Johnson JC, Sharp GC. Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings. Arthritis Rheum. 1999;42:899–909.

    Article  CAS  PubMed  Google Scholar 

  24. Sciascia S, Cuadrado MJ, Khamashta M, Roccatello D. Renal involvement in antiphospholipid syndrome. Nat Rev Nephrol. 2014;10:279–89.

    Article  CAS  PubMed  Google Scholar 

  25. Takizawa Y, Kanda H, Sato K, Kawahata K, Yamaguchi A, Uozaki H, et al. Polymyositis associated with focal mesangial proliferative glomerulonephritis with depositions of immune complexes. Clin Rheumatol. 2007;26:792–6.

    Article  PubMed  Google Scholar 

  26. Akpolat T, Akkoyunlu M, Akpolat I, Dilek M, Odabas AR, Ozen S. Renal Behçet’s disease: a cumulative analysis. Semin Arthritis Rheum. 2002;31:317–37.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

The authors are grateful to all colleagues who participated in the J-RBR (online Appendix). This study was supported in part by the committee of the Japanese Society of Nephrology, and by a Grant-in-aid for Intractable Renal Disease Research, Research on Rare and Intractable Diseases, Health and Labour Sciences Research Grants from the Ministry of Health, Labour, and Welfare of Japan.

Author information

Authors and Affiliations

  1. Department of Cardiology, Pulmonology, and Nephrology, Yamagata University School of Medicine, 2-2-2, Iida-Nishi, Yamagata, 990-9585, Japan

    Kazunobu Ichikawa & Tsuneo Konta

  2. Clinical Pharmacology and Therapeutics, Tohoku University Graduate School of Pharmaceutical Sciences, Sendai, Japan

    Hiroshi Sato

  3. Department of Pathology, Dokkyo Medical University Koshigaya Hospital, Koshigaya, Japan

    Yoshihiko Ueda

  4. Division of Nephrology, Kanazawa Medical University School of Medicine, Uchinada, Japan

    Hitoshi Yokoyama

Authors
  1. Kazunobu Ichikawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tsuneo Konta
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hiroshi Sato
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yoshihiko Ueda
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hitoshi Yokoyama
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Tsuneo Konta.

Ethics declarations

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee at which the studies were conducted (IRB approval number: the Japanese Society of Nephrology, No.32, Oct 25, 2014) and with the 1964 Helsinki declaration and its later amendments, or comparable ethical standards.

Conflict of interest

The authors have no conflicts of interest to declare.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 37 KB)

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ichikawa, K., Konta, T., Sato, H. et al. The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol 21, 1024–1029 (2017). https://doi.org/10.1007/s10157-017-1398-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10157-017-1398-5

Keywords

Search

Navigation